Can early-onset acquired demyelinating syndrome (ADS) hide pediatric Behcet's disease? A case report.
Behcet’s disease
acquired demyelinating syndromes (ADSs)
children
early onset
magnetic resonance imaging
Journal
Frontiers in pediatrics
ISSN: 2296-2360
Titre abrégé: Front Pediatr
Pays: Switzerland
ID NLM: 101615492
Informations de publication
Date de publication:
2023
2023
Historique:
received:
27
02
2023
accepted:
30
05
2023
medline:
10
7
2023
pubmed:
10
7
2023
entrez:
10
7
2023
Statut:
epublish
Résumé
Behcet's disease (BD) is a rare vasculitis characterized by multisystemic inflammation. Central nervous system (CNS) involvement is rare and heterogeneous, particularly in the pediatric population. A diagnosis of neuro-Behcet could be highly challenging, especially if neurological manifestations precede other systemic features; however, its timely definition is crucial to prevent long-term sequelae. In this study, we describe the case of a girl who, at 13 months of age, presented with a first episode of encephalopathy compatible with acute disseminated encephalomyelitis, followed, after 6 months, by a neurological relapse characterized by ophthalmoparesis and gait ataxia, in association with new inflammatory lesions in the brain and spinal cord, suggesting a neuromyelitis optica spectrum disorder. The neurological manifestations were successfully treated with high-dose steroids and intravenous immunoglobulins. In the following months, the patient developed a multisystemic involvement suggestive of Behcet's disease, characterized by polyarthritis and uveitis, associated with HLA-B51 positivity. The challenge presented by this unique case required a multidisciplinary approach involving pediatric neurologists, neuro-radiologists, and pediatric rheumatologists, with all of these specialists creating awareness about early-onset acquired demyelinating syndromes (ADSs). Given the rarity of this presentation, we performed a review of the literature focusing on neurological manifestations in BD and differential diagnosis of patients with early-onset ADS.
Identifiants
pubmed: 37425262
doi: 10.3389/fped.2023.1175584
pmc: PMC10327559
doi:
Types de publication
Case Reports
Langues
eng
Pagination
1175584Informations de copyright
© 2023 Pozzato, Dilena, Rogani, Beretta, Torreggiani, Lanni, Tozzo, Andreetta, Cavalcante, Triulzi, Martinelli Boneschi, Minoia and Filocamo.
Déclaration de conflit d'intérêts
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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